TABLE IV-114 Risk Factors for Active Tuberculosis Among Persons Who Have Been Infected With Tubercle Bacilli 25 page
appropriate step?
A. Aspirin 975 mg po daily
B. Measurement of erythrocyte sedimentation rate
C. Prednisone 60 mg daily
D. Referral for temporal artery biopsy
E. Referral for ultrasound of temporal artery
IX-45. A 54-year-old man is evaluated for cutaneous vasculitis and peripheral nephropathy. Because of concomitant renal dysfunction he undergoes kidney biopsy that shows glomerulonephritis. Cryoglobulins are demonstrated in the peripheral blood. Which of the following laboratory studies should be sent to determine the etiology?
A. Hepatitis B surface antigen
B. Cytoplasmic ANCA
C. Hepatitis C polymerase chain reaction (PCR)
D. HIV antibody
E. Rheumatoid factor
IX-46. A 54-year-old man is admitted for persistent lower abdominal and groin pain that began 7 months previously. Two months before his present admission, he required exploratory laparoscopy for acute abdominal pain and presumed cholecystitis. This revealed necrotic omental tissue and pericholecystitis necessitating omentectomy and cholecystectomy. However, the pain continued unchanged. He currently describes it as periumbilical and radiating into his groin and legs. It becomes worse with eating. The patient has also had episodic severe testicular pain, bowel urgency, nausea, vomiting, and diuresis. He has lost approximately 22.7 kg over the preceding 6 months. His past medical history is significant for hypertension that has recently become difficult to control.
Medications on admission include aspirin, hydrochlorothiazide, hydromorphone, lansoprazole, metoprolol, and quinapril. On physical examination, the patient appears comfortable. His blood pressure is 170/100 mmHg, his heart rate is 88 beats/min, and he is afebrile. He has normal first and second heart sounds without murmurs, and an S4 is present. There are no carotid, renal, abdominal, or
femoral bruits.
His lungs are clear to auscultation. Bowel sounds are normal. Abdominal palpation demonstrates minimal diffuse tenderness without rebound or guarding. No masses are present, and the stool is negative for occult blood. During the examination, the patient develops Raynaud’s phenomenon in his right hand that persists for several minutes. His neurologic examination is intact. Admission laboratory studies reveal an erythrocyte sedimentation rate of 72 mm/h, a BUN of 17 mg/dL, and a creatinine of 0.8 mg/dL. The patient has no proteinuria or hematuria. Tests for antinuclear antibodies, anti–double-stranded-DNA antibodies, and antineutrophil cytoplasmic antibodies are negative. Liver function tests are abnormal with an AST of 89 IU/L and an ALT of 112 IU/L. Hepatitis B surface antigen and e antigen are positive. Mesenteric angiography demonstrates small, beaded aneurysms of the superior and inferior mesenteric veins. What is the most likely diagnosis?
A. Hepatocellular carcinoma
B. Ischemic colitis
C. Microscopic polyangiitis
D. Mixed cryoglobulinemia
E. Polyarteritis nodosa
IX-47. An 18-year-old man is admitted to the hospital with acute onset of crushing substernal chest pain that began abruptly 30 minutes ago. He reports the pain radiating to his neck and right arm. He has otherwise been in good health. He currently plays trumpet in his high school marching band but does not participate regularly in aerobic activities. On physical examination, he is diaphoretic and tachypneic. His blood pressure is 100/48 mmHg and heart rate is 110 beats/min. His cardiovascular examination shows a regular rhythm but is tachycardic. A II/VI holosystolic murmur is heard best at the apex and radiates to the axilla. His lungs have bilateral rales at the bases. The electrocardiogram demonstrates 4 mm of ST elevation in the anterior leads. On further questioning regarding his past medical history, he recalls having been told that he was hospitalized for some problem with his heart when he was 2 years old. His mother, who accompanies him, reports that he received aspirin and γ-globulin as treatment. Since that time, he has required intermittent follow-up with echocardiography. What is the most likely cause of this patient’s acute coronary syndrome?
A. Dissection of the aortic root and left coronary ostia
B. Presence of a myocardial bridge overlying the left anterior descending artery
C. Stenosis of a coronary artery aneurysm
D. Vasospasm following cocaine ingestion
E. Vasculitis involving the left anterior descending artery
IX-48. Which of the following is required for the diagnosis of Behçet’s disease?
A. Large-vessel vasculitis
B. Pathergy test
C. Recurrent oral ulceration
D. Recurrent genital ulceration
E. Uveitis
IX-49. A 25-year-old female presents with a complaint of painful mouth ulcerations. She describes these lesions as shallow ulcers that last for 1–2 weeks. The ulcers have been appearing for the last 6 months. For the last 2 days, the patient has had a painful red eye. She has had no genital ulcerations, arthritis, skin rashes, or photosensitivity. On physical examination, the patient appears well developed and in no distress. She has a temperature of 37.6°C (99.7°F), heart rate of 86 beats/min, blood pressure of 126/72 mmHg, and respiratory rate of 16 breaths/min. Examination of the oral mucosa reveals two shallow ulcers with a yellow base on the buccal mucosa. The ophthalmologic examination is consistent with anterior uveitis. The cardiopulmonary examination is normal. She has no arthritis, but medially on the right thigh there is a palpable cord in the saphenous vein. Laboratory studies reveal an erythrocyte sedimentation rate of 68 seconds. White blood cell count is 10,230/μL with a differential of 68% polymorphonuclear cells, 28% lymphocytes, and 4% monocytes. The antinuclear antibody and anti-dsDNA antibody are negative. C3 is 89 mg/dL, and C4 is 24 mg/dL. What is the most likely diagnosis?
A. Behçet’s syndrome
B. Cicatricial pemphigoid
C. Discoid lupus erythematosus
D. Sjögren’s syndrome
E. Systemic lupus erythematosus
IX-50. What is the best initial treatment for the patient in question IX-49?
A. Colchicine
B. Intralesional interferon α
C. Systemic glucocorticoids and azathioprine
D. Thalidomide
E. Topical glucocorticoids including ophthalmic prednisolone
IX-51. Relapsing polychondritis may be a primary disease or may be associated with other rheumatologic diseases. All of the following conditions are associated with relapsing polychondritis EXCEPT:
A. Myelodysplastic syndrome
B. Primary biliary cirrhosis
C. Scleroderma
D. Spondyloarthritides
E. Systemic lupus erythematosus
IX-52. A 47-year-old man is evaluated for 1 year of recurrent episodes of bilateral ear swelling. The ear is painful during these events, and the right ear has become floppy. He is otherwise healthy and reports no illicit habits. He works in an office and his only sport is tennis. On examination, the left ear has a beefy red color, and the pinna is tender and swollen; the earlobe appears minimally swollen but is neither red nor tender. Which of the following is the most likely explanation for this finding?
A. Behçet’s syndrome
B. Cogan’s syndrome
C. Hemoglobinopathy
D. Recurrent trauma
E. Relapsing polychondritis
IX-53. A 25-year-old African-American woman is evaluated for bilateral hilar lymphadenopathy found on a routine chest radiograph performed before a laparoscopic cholecystectomy. She undergoes mediastinoscopy, and multiple noncaseating granulomas are identified in her lymph nodes. All of the following may explain this finding EXCEPT:
A. Alveolar proteinosis
B. Atypical mycobacteria
C. Beryllium exposure
D. Histoplasmosis
E. Malignancy
F. Sarcoidosis
IX-54. A 34-year-old woman has a history of cutaneous sarcoidosis that has been managed with hydroxychloroquine for the last 5 years. After an episode of right flank pain and hematuria, she is
diagnosed with renal calculus. Which of the following statements regarding her renal calculus is true?
A. Exogenous vitamin D and sunlight exposure in patients with sarcoidosis may exacerbate hypercalcemia and associated renal calculus.
B. Hypercalcemia is rare in sarcoidosis and is unlikely to contribute to the patient’s calculus.
C. Hypercalcemia in sarcoidosis occurs through increased production of 25-dihydroxyvitamin D by the skin.
D. If she is to begin therapy with oral calcium to treat the renal stone, a 24-hour urine phosphate should be obtained before and after initiation of therapy.
E. None of the above.
IX-55. All of the following agents have been shown to improve symptoms or function in patients with sarcoidosis EXCEPT:
A. Etanercept
B. Hydroxychloroquine
C. Infliximab
D. Methotrexate
E. Prednisone
IX-56. All of the following statements regarding the clinical manifestations of sarcoidosis are true EXCEPT:
A. Cardiac involvement occurs in 25% of patients.
B. Eye involvement is typically anterior uveitis.
C. Liver involvement is typically manifest by elevation of alkaline phosphatase.
D. Lung involvement occurs in over 90% of cases.
E. Skin involvement occurs in approximately one-third of patients.
IX-57. You are seeing a 56-year-old woman for complaints of joint pain and stiffness. All of the following signs or symptoms would be indicative of inflammatory causes of arthritis EXCEPT:
A. Elevations in erythrocyte sedimentation rate
B. Fatigue, fever, or weight loss
C. Persistence for longer than 6 weeks
D. Presence of soft-tissue swelling around affected joints
E. Prolonged morning stiffness
IX-58. A 22-year-old man is seen for a shoulder injury that occurred while pitching in a baseball game. He describes feeling a snap then acute pain in the shoulder of his left arm while throwing the ball. Which of the following findings would be most concerning for a tear of one of the rotator cuff muscles?
A. Inability to hold the arm at 90° following passive abduction
B. Inability to actively raise the arm more than 90° with forward flexion
C. Pain with palpation over the bicipital groove while rotating the arm internally and externally
D. Pain with palpation while applying pressure anteriorly along the joint and rotating the arm
internally and externally
E. Pain with passive abduction of the arm
IX-59. A 62-year-old white male presents with a chief complaint of right knee pain and swelling. Past medical history is significant for obesity with a body mass index (BMI) of 34 kg/m2, diet-controlled Type 2 diabetes mellitus, and hypertension. His medications include hydro-chlorothiazide and acetaminophen as needed for pain. Physical examination is remarkable for a moderately sized effusion of the right knee, with range of motion limited to 90° of flexion and 160° of extension. There is minimal warmth and no redness. He has crepitus with range of motion. With weight bearing, he has outward bowing of the legs bilaterally. A radiogram of the right knee shows osteophytes and joint space narrowing. Which of the following is the most likely finding on joint fluid examination?
A. A Gram stain showing gram-positive cocci in clusters
B. A white blood cell count of 1110/μL
C. A white blood cell count of 22,000/μL
D. Positively birefringent crystals on polarizing light microscopy
E. Negatively birefringent crystals on polarizing light microscopy
IX-60. A 62-year-old woman presents complaining of hand pain bilaterally that has been gradually progressive over the past year. She has previously worked as a seamstress in a factory making gloves for more than 35 years. You suspect osteoarthritis. All of the following factors on history or physical examination are characteristic of this diagnosis EXCEPT:
A. Evidence of bilateral swelling and warmth affecting the wrists only
B. Joint space narrowing and osteophytes at the proximal and distal interphalangeal joints on x-ray
C. Pain that becomes worse when preparing meals
D. Presence of Heberden’s nodes
E. Stiffness that is worse after brief periods of rest with occasional locking of the more affecting joints
IX-61. A 73-year-old woman with a medical history of obesity and diabetes mellitus presents to your clinic complaining of right knee pain that has been progressive and is worse with walking or standing. She has taken over-the-counter nonsteroidal anti-inflammatory drugs without relief. She wants to know what is wrong with her knee and what may have caused it. X-rays are performed and reveal cartilage loss and osteophyte formation. Which of the following represents the most potent risk factor for the development of osteoarthritis?
A. Age
B. Gender
C. Genetic susceptibility
D. Obesity
E. Previous joint injury
IX-62. A 53-year-old man presents to your clinic complaining of bilateral knee pain. He states that the pain worsens with walking and is not present at rest. He has been experiencing knee pain intermittently for many months and has had no relief from over-the-counter analgesics. He has a history of
hypertension and obesity. When he was in high school and college, he played football and basketball. Which of the following represents the best initial treatment strategy for this patient?
A. Avoidance of walking for several weeks
B. Light daily walking exercises
C. Low-dose, long-acting narcotics
D. Oral steroid pulse
E. Weight loss
IX-63. A 74-year-old man is seen by his primary care provider 6 weeks following an acute gout attack. He has a prior history of gout presenting similarly on two prior occasions within the past 6 months. His past medical history is significant for congestive heart failure, hypercholesterolemia, and stage III chronic kidney disease. He is taking pravastatin, aspirin, furosemide, metolazone, lisinopril, and metoprolol XL. His glomerular filtration rate is 38 mL/min, creatinine is 2.2 mg/dL, and uric acid level is 9.3 mg/dL. He is wondering if there is any therapy that might lessen his likelihood of repeated gout attacks. Which of the following medication regimens is most appropriate for the treatment of this patient?
A. Allopurinol 800 mg daily
B. Colchicine 0.6 mg bid
C. Febuxostat 40 mg daily
D. Indomethacin 25 mg twice daily
E. Probenecid 250 mg twice daily
IX-64. A 64-year-old man with congestive heart failure presents to the emergency department complaining of acute onset of severe pain in his right foot. The pain began during the night and awoke him from a deep sleep. He reports the pain to be so severe that he could not wear a shoe or sock to the hospital. His current medications are furosemide 40 mg twice daily, carvedilol 6.25 mg twice daily, candesartan 8 mg once daily, and aspirin 325 mg once daily. On examination, he is febrile to 38.5°C (101.3°F). The first toe of the right foot is erythematous and exquisitely tender to touch. There is significant swelling and effusion of the first metatarsophalangeal joint on the right foot. No other joints are affected. Which of the following findings would be expected on arthrocentesis?
A. Glucose level of less than 25 mg/dL
B. Positive Gram stain
C. Presence of strongly negatively birefringent needle-shaped crystals under polarized light microscopy
D. Presence of weakly positively birefringent rhomboidal crystals under polarized light microscopy
E. White blood cell (WBC) count greater than 100,000/μL
IX-65. A 24-year-old woman is admitted to the hospital with symptoms of fever and a swollen, painful right knee. About 3 weeks prior to the current syndrome, the patient had systemic symptoms including fevers, chills, and migratory joint pains affecting the hands, wrists, knees, hips, and ankles. At that time, she noticed a few small papules on her upper chest and hands. These have subsequently resolved. She has no significant past medical history. She currently works as a landscape designer and does not recall
any recent tick or insect bites. Her only medication is an oral contraceptive. She is unmarried and has multiple sexual partners. On physical examination, the patient has a temperature of 38.4°C (101.2°F), heart rate of 124 beats/min, respiratory rate of 24 breaths/min, and blood pressure of 102/68 mmHg. Her right knee demonstrates redness, warmth, swelling, and pain with movement. An arthrocentesis demonstrates a white blood cell count of 66,000/μL (90% neutrophils). No crystals or organisms are seen. Which of the following would be most likely to yield the correct diagnosis?
A. Bacterial cultures of the cervix
B. Bacterial cultures of the synovial fluid
C. Blood cultures
D. IgG directed against Borrelia burgdorferi
E. Rheumatoid factor
IX-66. A 66-year-old woman with a history of rheumatoid arthritis and frequent attacks of pseudogout in her left knee presents with night sweats and a 2-day history of left knee pain. Her medications include methotrexate 15 mg weekly, folate 1 mg daily, prednisone 5 mg daily, and ibuprofen 800 mg three times daily as needed for pain. On physical examination, her temperature is 38.6°C (101.5°F), heart rate is 110 beats/min, blood pressure is 104/78 mmHg, and oxygen saturation is 97% on room air. Her left knee is swollen, red, painful, and warm. With 5° of flexion or extension, she develops extreme pain. She has evidence of chronic joint deformity in her hands, knees, and spine. Peripheral white blood cell (WBC) count is 16,700 cells/μL with 95% neutrophils. A diagnostic tap of her left knee reveals 168,300 WBCs per μL and 99% neutrophils, and diffuse needle-shaped birefringent crystals are present. Gram stain shows rare gram-positive cocci in clusters. Management includes all of the following EXCEPT:
A. Blood cultures
B. Glucocorticoids
C. Needle aspiration of joint fluid
D. Orthopedic surgery consult
E. Vancomycin
IX-67. A 42-year-old woman is seen in her primary care doctor’s office complaining of diffuse pains and fatigue. She has a difficult time localizing the pain to any particular joint or location, but reports that it affects her upper and lower extremities, neck, and hips. It is described as achy and 10 out of 10 in intensity. She feels that her joints are stiff but does not notice that it is worse in the morning. The pain has been present for the past 6 months and is increasing in intensity. She has tried both over-the-counter ibuprofen and acetaminophen without significant relief. The patient feels as if the pain is interfering with her ability to get restful sleep and is making it difficult for her to concentrate. She has missed multiple days of work as a waitress and fears that she will lose her job. There is a medical history of depression and obesity. The patient currently is taking venlafaxine sustained release 150 mg daily. She has a family history of rheumatoid arthritis in her mother. She smokes 1 pack of cigarettes daily. On physical examination vital signs are normal. Body mass index is 36 kg/m2. Joint examination demonstrates no erythema, swelling, or effusions. There is diffuse pain with palpation at the insertion points of the suboccipital muscles, at the midpoint of the upper border of the trapezius muscle, along the second costochondral junction, at the lateral epicondyles, and along the medial fat pad of the knees. All
of the following statements regarding the cause of this patient’s diffuse pain syndrome are true EXCEPT:
A. Cognitive dysfunction, sleep disturbance, anxiety, and depression are common comorbid neuropsycho-logical conditions.
B. Pain in this syndrome is associated with increased evoked pain sensitivity.
C. Pain in this syndrome is often localized to specific joints.
D. This syndrome is present in 2–5% of the general population, but increases in prevalence to 20% or more of patients with degenerative or inflammatory rheumatic disorders.
E. Women are nine times more likely than men to be affected by this syndrome.
IX-68. A 36-year-old woman presents to your office with diffuse pain throughout her body associated with fatigue, insomnia, and difficulty concentrating. She finds the pain difficult to localize, but reports that it is 7–8 out of 10 in intensity and is not relieved by nonsteroidal anti-inflammatory medications. She has a long-standing history of generalized anxiety disorder and is treated with sertraline 100 mg daily as well as clonazepam 1 mg twice daily. On examination, she has pain with palpation at several musculoskeletal sites. Her laboratory examination demonstrates a normal complete blood count, basic metabolic panel, erythrocyte sedimentation rate, and rheumatoid factor. You diagnose her with fibromyalgia. All of the following therapies are recommended as part of the treatment plan for fibromyalgia EXCEPT:
A. An exercise program that includes strength training, aerobic exercise, and yoga
B. Cognitive-behavioral therapy for insomnia
C. Milnacipran
D. Oxycodone
E. Pregabalin
IX-69. A 53-year-old woman presents to your clinic complaining of fatigue and generalized pain that have worsened over 2 years. She also describes irritability and poor sleep, and is concerned that she is depressed. She reveals that she was recently separated from her husband and has been stressed at work. Which of the following elements in her history and physical examination would meet American College of Rheumatology criteria for diagnosis of fibromyalgia?
A. Diffuse chronic pain and abnormal sleep
B. Diffuse pain without other etiology and evidence of major depression
C. Major depression, life stressor, chronic pain, and female gender
D. Major depression and pain on palpation at 6 of 18 tender point sites
E. Widespread chronic pain and pain on palpation at 11 of 18 tender point sites
IX-70. A 42-year-old man is found to have the following finding on a physical examination (Figure IX-70). All of the following conditions are associated with this finding EXCEPT:
FIGURE IX-70 (Reprinted from the Clinical Slide Collection on the Rheumatic Diseases, Copyright 1991, 1995. Used by permission of the American College of Rheumatology.)
A. Chronic obstructive pulmonary disease
B. Cyanotic congenital heart disease
C. Cystic fibrosis
D. Hepatocellular carcinoma
E. Hyperthyroidism
IX-71. A 64-year-old woman sees her primary care physician complaining of hip pain for about 1 week. She localizes the pain to the lateral aspect of her right hip and describes it as sharp. It is worse with movement, and she finds it difficult to lie on her right side. The pain began soon after the patient planted her garden. She has a medical history of obesity, osteoarthritis of the knees, and hypertension. Her medications include losartan 50 mg daily and hydrochlorothiazide 25 mg daily. For the pain, she has taken ibuprofen 600 mg as needed with mild to moderate relief of pain. On physical examination, the patient is not febrile and her vital signs are unremarkable. On examination of the hip, pain is elicited with external rotation and resisted abduction of the hip. Direct palpation over the lateral aspect of the upper portion of the femur near the hip joint reproduces the pain. What is the most likely diagnosis in this patient?
A. Avascular necrosis of the hip
B. Iliotibial band syndrome
C. Meralgia paresthetica
D. Septic arthritis
E. Trochanteric bursitis
IX-72. A 32-year-old woman is seen in the clinic with a complaint of left knee pain. She enjoys running long distances and is currently training for a marathon. She is running on average 30–40 miles weekly. She currently is experiencing an aching pain on the lateral aspect of her left knee. There is a burning sensation that also continues up the lateral aspect of her thigh. She denies any injury to her knee, and she has not felt that it was hot or swollen. She is otherwise healthy and takes no medications other than herbal supplements. Physical examination of the knee reveals point tenderness over the lateral femoral condyle that is worse with flexing the knee. The patient is asked to lie on her right side with her right knee and hip flexed at 90°. Her left leg is extended at the hip and slowly lowered into adduction behind the bottom leg, reproducing the patient’s left knee pain. All of the following treatments can be
recommended for this patient EXCEPT:
A. Assessment of the patient’s running shoes to ensure a proper fit
B. Glucocorticoid injection so as not to interfere with the patient’s continued preparation for the upcoming marathon
C. Ibuprofen 600–800 mg every 6 hours as needed for pain
D. Referral for physical therapy
E. Referral for surgical release if conservative therapy fails
IX-73. A 58-year-old female presents complaining of right shoulder pain. She does not recall any prior injury but notes that the shoulder has been getting progressively stiffer over the last several months. She previously had several episodes of bursitis of the right shoulder that were treated successfully with NSAIDs and steroid injections. The patient’s past medical history is also significant for diabetes mellitus, for which she takes metformin and glyburide. On physical examination, the right shoulder is not warm or red but is tender to touch. Passive and active range of motion is limited in flexion, extension, and abduction. A right shoulder radiogram shows osteopenia without evidence of joint erosion or osteophytes. What is the most likely diagnosis?
A. Adhesive capsulitis
B. Avascular necrosis
C. Bicipital tendinitis
D. Osteoarthritis
E. Rotator cuff tear
IX-74. A 32-year-old woman presents to the clinic with right thumb and wrist pain that has worsened over several weeks. She has pain when she pinches her thumb against her other fingers. Her only other history is that she is a new mother with an 8-week-old infant at home. On physical examination she has mild swelling and tenderness over the radial styloid process, and pain is elicited when she places her thumb in her palm and grasps it with her fingers. A Phalen maneuver is negative. Which condition is most likely?
A. Carpal tunnel syndrome
B. De Quervain’s tenosynovitis
C. Gouty arthritis of the first metacarpophalangeal joint
D. Palmar fasciitis
E. Rheumatoid arthritis
ANSWERS
IX-1. The answer is A. (Chap. 314) The innate immune system is phylogenetically the oldest form of immunologic defense system, inherited from invertebrates. This defense system uses germ line–encoded proteins to recognize pathogen-associated molecular patterns. Cells of the innate immune system include macrophages, dendritic cells, and natural killer lymphocytes. The critical components of the innate immune system include recognition by germ line–encoded host molecules, recognition of key
microbe virulence factors but not recognition of self molecules, and nonrecognition of benign foreign molecules or microbes. Adaptive immunity is found only in vertebrate animals and is based on the generation of antigen receptors on T and B lymphocytes by gene rearrangements, such that individual T or B cells express unique antigen receptors on their surface capable of recognizing diverse environmental antigens.
IX-2. The answer is A. (Chap. 314) Complement activity, which results from the sequential interaction of a large number of plasma and cell membrane proteins, plays an important role in the inflammatory response. The classic pathway of complement activation is initiated by an antibody–antigen interaction. The first complement component (C1, a complex composed of three proteins) binds to immune complexes with activation mediated by C1q. Active C1 then initiates the cleavage and concomitant activation of components C4 and C2. The activated C1 is destroyed by a plasma protease inhibitor termed C1 esterase inhibitor. This molecule also regulates clotting factor XI and kallikrein. Patients with a deficiency of C1 esterase inhibitor may develop angioedema, sometimes leading to death by asphyxia. Attacks may be precipitated by stress or trauma. In addition to low antigenic or functional levels of C1 esterase inhibitor, patients with this autosomal-dominant condition may have normal levels of C1 and C3 but low levels of C4 and C2. Danazol therapy produces a striking increase in the level of this important inhibitor and alleviates the symptoms in many patients. An acquired form of angioedema caused by a deficiency of C1 esterase inhibitor has been described in patients with autoimmune or malignant disease.